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KMID : 0363219920300040558
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Korean Journal of Dermatology
1992 Volume.30 No. 4 p.558 ~ p.563
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Neurocutaneous Melanosis
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Abstract
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Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital pigmented nevi and a benign or malignant pigment cell tumor of the leptomeninges. The syndrome is thought to represent an error
in
the
morphogenesis of the embryonal neuroectoderm. Even in the absence of melanoma, symptomatic neurocutaneous melanosis has extremely poor prognosis.
We report a case of neurocutaneous melanosis in 38-year-old male. He was noted at birth to have a giant pigmented nevus on the bathing trunk area. He presneted with severe headache. Brain CT scan was performed and 4×4cm sized well demarcated
contrast
enhancd mass was seen in the right frontal lobe. Histopathologically, the tumor was composed of malignant melanoma cells. Multiple skin biopsies were performed on the giant pigmented nevus and small nevi. All of the specimens revealed patterns
typical
of compound nevi.
Dermatologists following patients with large or multiple congenital pigmnted nevi should be a ware of this condition to aid in prompt diagnosis.
(Kor J Dermatol 1992;30(4):558-563)
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